Although meat from a 12-year-old Texas beef cow with mad cow disease never entered the food supply, critics of the Agriculture Department said the twisted, seven-month-long tale of this animal highlighted bureaucratic missteps and weaknesses in the food safety system.

Public confidence wasn't enhanced last week when the USDA announced that a private veterinarian had "forgotten" about a brain tissue sample he took in April. It came from another cow now suspected of having had the brain-wasting disease bovine spongiform encephalopathy. Definitive tests are under way.

Watchdog groups awarded barely passing marks to the department for its handling of the Texas cow, which turned up dead at a Waco packing plant Nov. 15. The USDA finally confirmed the mad cow case June 10 after multiple tests.

"USDA gets a D or D minus," said Caroline Smith Dewaal of the Washington-based advocacy group, Center for Science in the Public Interest. "The best thing that came out of this is the work of the inspector general."

It was the department's in-house watchdog, Inspector General Phyllis Fong, who skirted the USDA hierarchy by ordering retesting with a different method more than six months after a routine second-round test, known as the immunohistochemistry (IHC) test, proved negative for BSE.

Agriculture Secretary Mike Johanns, who assumed office in January, has said he neither knew about nor authorized the retesting by the National Veterinary Services Laboratories in Ames, Iowa.

Just why Fong, a lawyer who grew up in Hawaii, acted in such a forthright, hierarchy-dodging manner has puzzled many involved in the industry.

In a statement, her office said the retesting was prompted by a review of "voluminous records" showing an unusual pattern of conflicting test results in the case. Industry sources say there is speculation that she responded to concern expressed in scientific circles.

Two early tests, one reportedly conducted at Texas A&M University and a second in Ames, produced conflicting results -- one inconclusive, one negative.

Unbeknownst to Fong and the public, Ames researchers had also used an experimental rapid version of the IHC test on brain tissue from the Texas cow. That proved positive for BSE, but staff members thought the result was technically flawed and the USDA didn't disclose until just recently that the Ames lab had conducted the experimental test.

Months later, Fong stepped in and ordered more tests. A "Western blot" test proved positive, as did later tests at a lab in Weybridge, England.

Finally in June, two days after the Weybridge lab confirmed the mad cow case, a chastened USDA announced that in addition to the routine IHC test, it was adopting the Western blot procedure whenever an initial "BioRad" screening test points to possible BSE. In addition, backup tests will now be conducted at Britain's national veterinary laboratory in Weybridge when earlier test results conflict or are inconclusive.

All this sounded familiar to Consumers Union.

In February, the nonprofit public interest group that publishes Consumer Reports urged the USDA to take those same steps in regard to the Texas cow, noted Michael Hansen, a senior researcher with the organization.

In hindsight, the March 18 response to Consumers Union by Jere Dick, associate deputy administrator of USDA's animal health policy and programs, smeared egg clearly across the department's collective face.

Referring to the Texas cow, Dick wrote:

"We are confident in the expertise of USDA's laboratory technicians conducting BSE testing and do not feel that such confirmatory testing by the Weybridge laboratory is generally necessary, nor would the use of the Western blot test have enhanced the result of our November 2004 testing."

The opposite turned out to be true.

Sen. Tom Harkin, D-Iowa, noted the obvious in a July 7 letter to Johanns:

"Without the prompting of the [inspector general's office] , we still would not know that the brain sample in question tests BSE-positive."

While Americans should have no misgivings about eating steaks and hamburgers, Harkin said, "there is a limit to how much of its own errors, inconsistencies and lack of transparency USDA can reasonably expect consumers to abide and still have confidence in the safety of beef."

Food supply kept safe

All the criticism might obscure the fact that meat from diseased livestock has been kept out of the food supply. BSE is carried by hard-to-destroy protein prions, which scientists believe can cause a rare human disease -- variant Creutzfeldt-Jakob -- if found in beef.

The food supply has been protected by a ban for human consumption of tissue most prone to prion contamination, including beef brain, scrapings from the spinal column, and a small section of the intestine.

USDA's animal health officials were dealing with a highly unusual situation, said Keith Belk, a Colorado State University animal science professor who closely follows the BSE issue.

"There clearly were some mistakes made, but this was a pretty unique cow," Belk said. "She had the disease, and it incubated for a long time. But it apparently received a light dose of prions that were very sporadically spread" in the brain tissue.

The tissue sample, a pie-slice-shaped sliver taken from the obex, a slight bulge in the brain stem, had so few BSE prions at some points that a British expert, Dr. Danny Matthews of Weybridge, said tests easily could have missed them.

Matthews has said BSE is becoming rarer and more difficult to detect because of effective bans on tainted cattle feeds, which are believed to spread the disease.

Some of the missteps, ironically, were caused by USDA staff doing exactly what they were supposed to do -- faithfully following measures that go far beyond international standards, Belk said in a call from Fort Collins, Colo.

"They appeared to have made some significant errors, which unfortunately made them appear foolish," Belk said. "Most scientists around the world would have argued that they should have run both the Western blot and IHC tests. I think they would have liked to have done so; but because of policy in place, their hands were tied."

Under lab rules in effect in November, the USDA staff in Ames was not permitted to carry out any other type of test after it received a negative result from the second round of testing, using the IHC method.

Communication miscues haven't helped the USDA's image in its handling of the Texas case.

In June, the USDA finally confirmed that the yellow to cream-colored cow had arrived dead at a meatpacking plant, not a dog food plant as originally reported.

And it had not been a downer -- a live but nonwalking animal -- as described by officials for nearly seven months. Making matters worse, the initial description of the cow as an Angus was wrong, and some of its high-risk body parts were tossed in a bin with those of other cattle, creating the need to run a number of DNA tests.

Dewaal also criticized the successful effort to keep the name and location of the ranch that produced the Brahman cross-bred cow secret. The ranch has been speculated to be somewhere in Southeast Texas.

"Who are they protecting?" she asked. "The only person it protects is the rancher."

But officials like Carla Everett, a spokeswoman with the Texas Animal Health Commission, say that maintaining confidentiality is necessary to prevent such operations from being needlessly stigmatized and to ensure future cooperation from ranches to which other diseased animals might be traced.

Owning up

Although chastened, the USDA said it had no choice but to treat the Texas cow case as it had.

"It was handled within our protocol with few exceptions," said Jim Rogers, a spokesman for the department's Animal and Plant Health Inspection Service.

But the department is owning up to some mistakes.

Johanns has acknowledged that some things should have been done differently, Rogers said. Among them: Animal parts from different cattle should not have been commingled as they were at the Waco pet food plant; the Ames laboratory should not have run an experimental test while running the official test; and the lab made a verbal disclosure, but it should have presented a written report.

Whatever steps the USDA takes, it could find itself in a no-win situation, Rogers said.

In November, Western blot was not part of the testing protocol because it was not deemed appropriate in that situation.

"Let's go back in time and say we had conducted Western blot," Rogers said. "We would have critics saying we had stepped outside our protocol."

In fact, the National Cattlemen's Beef Association publicly criticized the inspector general for stepping out of the testing routine -- just before it became clear that her irregular action had turned up the positive BSE finding.

Reinforcing experts' description of the Texas cow as an unusual case, Rogers noted that after Fong requested that Western blot be used on the brain sample, "they tested it 10 times -- five of which were negative and five positive."

The results could be far different depending on what part of the sample was examined because of the erratic spread of BSE prions. Scientists believe that these submicroscopic flecks of protein spread BSE, a rare but always fatal disease discovered in 1986, through feed contaminated with rendered cattle parts.

The infected Texas cow was born before such tainted feed was banned in the United States in 1997. Ninety-seven percent of beef now consumed by Americans comes from cattle born after the ban, according to the National Cattlemen's Beef Association.

By comparison, Japan did not ban such feed until 2001, possibly contributing to the 20 BSE cases there -- and a heightened popular distrust of its food security program, Belk said. That lack of public confidence prompted testing of every animal going to slaughter and a continued reluctance to end a 19-month ban on U.S. beef imports.

While such feed is banned for cattle, it can still be fed to pigs and chickens, which cannot contract BSE.

But some scientists and consumer groups, fearing mislabeling of sacks or other human errors, have urged the Food and Drug Administration, which regulates the feed industry, to act on its own January 2004 recommendation and ban it for poultry and pigs, too.

One U.S. case

Of the 153 worldwide cases of a fatal human ailment believed caused by BSE-contaminated beef products, the only victim of variant Creutzfeldt-Jakob disease in the United States was a Florida woman who had lived in Britain when such tainted food was available.

Six months after the first U.S. case in a Canadian-born dairy cow was discovered in December 2003, scientists said BSE was probably present at low levels in the U.S. herd, and the USDA launched a widespread surveillance effort. The beef industry notes that the Texas animal was the only cow found with BSE after 419,113 high-risk animals have been tested.

In the most recent case, involving a cow that was euthanized after severe calving problems whose test samples were forgotten about, the carcass was incinerated. The Texas and Washington state cows were discovered before they entered the food chain, reinforcing the government and industry's insistence that world-class firewalls have kept U.S. consumers protected.

After the Texas cow arrived dead at a Waco packinghouse, it was trucked across town to a dog food plant, where test samples were taken. The animal's body, kept segregated, was incinerated at Texas A&M University.

Unfortunately, by then its parts were commingled with three other cattle, making the difficult task of tracing a suspect cow without a mandatory national animal identification program even harder. This was seen as another blunder by authorities.

Hansen of Consumers Union complained that little is known publicly about the 419,113 tested cattle -- or many others with central nervous system problems that were never examined.

He cited an August 2004 audit by the inspector general's office as saying that only a fraction of the highest-risk cattle -- erratic-acting animals that tested negative for rabies -- were not given the rapid BioRad test for BSE.

In fiscal 2003, for example, 108 Texas cattle were tested and found clean for rabies, but only 29 of this high-risk group were checked for BSE. In South Dakota, 81 tested cattle were negative for rabies, but none were given BSE tests.

Rogers conceded that there was a "mix-up" in states where testing guidelines were not clearly understood. The confusion has been remedied, and by late September, all animal inspectors understood that such high-risk cattle must be tested for BSE, he said.

Call for tracking

Chief among complaints by watchdog groups like Consumers Union and the Center for Science in the Public Interest is that the United States -- unlike the European Union, Japan, Canada, Australia and New Zealand -- still has no mandatory national identification program for tracking cattle. An ID program would help quickly trace the origins of a contaminated cow.

As the mad-cow story developed this summer, the National Cattlemen's Beef Association got a head start on Johanns by saying it was starting its own animal ID system and hoped to have it operational in January 2007.

The association is seeking to have a system that would eventually be taken over as the national program. It envisions one that would ensure confidentiality of certain proprietary data that cattle operations and others want withheld for competitive reasons.

"For example, Cargill doesn't want Tyson to know where they purchase cattle," said Belk of Colorado State, referring to major beef processing companies.

And ranchers, some of whom have run cattle for generations, are worried about consumer lawsuits, he said.

"If you can determine where something comes from, it opens the door to liability," Belk said.

Johanns had announced that the government's mandatory ID system won't be up and running until 2009.

"I think the system is flawed as long as we don't have a national mandatory system for tracking cows," said Dewaal, who noted that Canada went from a voluntary to a mandatory system in one year. "We may be better at finding the one infected animal but not others similarly exposed to the disease.

"And we are surprised by the extraordinary delay."

Consumers, ever more concerned about food safety, are putting money where their mouths are and demanding beef traceability.

In Texas, chains such as United Market Street, Whole Foods and Central Market are offering source-verified beef -- meat whose herd or ranch origin is spelled out to consumers.

In doing so, the market will push for an animal ID system to be implemented before 2009, Belk predicted. And source verifiability might be needed to re-establish lost export markets, he said.

And such traceability carries plenty of benefits.

Some systems could go beyond a mandatory animal ID system, Belk said, providing useful information: Is the animal free of antibiotics? Was it given vitamin E for a certain antioxidant level?

The producer could also receive feedback on whether livestock management techniques led to more tender cuts, for example.

"It's not all related to safety," Belk said.

BSE timeline

• Nov. 11, 2004

A no longer productive 12-year-old beef cow is sold at a Texas auction.

• Nov. 15, 2004

A cattle buyer ships the cow and others to H&B Packing Co. in Waco, where it arrives dead. The animal is transported across town to Champion Pet Food Co., where its head is severed and shipped to a laboratory at Texas A&M University in College Station.

• Nov. 18, 2004

Results of two Bio Rad rapid tests are announced by the U.S. Department of Agriculture as "inconclusive," meaning positive for this error-prone, sensitive method of determining bovine spongiform encephalopthy, or BSE. This prompts rounds of confirmatory tests.

• Nov. 23, 2004

Results are negative on brain tissue sent to the National Veterinary Services Laboratories in Ames, Iowa, for two immunohistochemistry (IHC) tests. A positive result four days earlier from an experimental rapid IHC test is not released because researchers believe that it was technically flawed.

• June 10, 2005

The USDA announces that a Western blot test ordered by the department's inspector general without Agriculture Secretary Mike Johann's knowledge is positive for BSE.

• June 24, 2005

Britain's national veterinary laboratory gets positive results using both IHC and Western blot tests. Johanns also discloses British confirmation that the Ames' experimental IHC test was positive for BSE.

• July 11, 2005

Sixty-seven cattle from the infected cow's herd test negative for BSE with the Bio-Rad method. Investigators are still trying to track down the infected cow's last two calves.

DR. CLIFFORD: "Basically the IHC test, besides looking at location of thebrain stem you're also doing a staining technique to identify abnormal prionproteins. In this case they had some staining, but the staining did notmatch up with what they would typically see in a BSE case. It didn't havethe normal distribution it would see within the samples. So basically that'swhy the request for doing additional testing, and that's why we're sendingit to Weybridge as well."

DR. CLIFFORD: "There was some staining present. But it did not match anormal pattern, and we're taking through that to do additional tests inadditional parts of the brain stem to try to see if we can find a normalstaining pattern as well as sending that sample to Weybridge to run againsttheir IHC."

PERCEPTION OF UNCONVENTENTIONAL SLOW VIRUS DISEASES OF ANIMALS IN THE USA

1985 The Stetsonville outbreak (farmer's name: Brecke). In addition to thedowner cows and horses Brecke's mink recieved a cereal supplement.Hartsough's view was that this would contain bone meal and would be from acommercial source. If this were so and it was contaminated with a TME agentwhy were no other ranches affected?

Many mink ranches now feed a commerical pelleted diet. Brecke was equippedto process LARGE CARCASSES USING A CRUSHER/MIXER WHICH COULD ACCOMMODATE AWHOLE COW!

snip...

Dead mink go for rendering but are used only in poultry feed.

A commercial mink ranch was visited. This was Johny Werth's, Capitol FurFarm comprising 1400 breeding females. The feed is bought in from acommercial supplier in the form of frozen packs of ''poultry'', ''fish'',''dried egg'' or ''tripe''. A commercial mink cereal supplement is used andcontains ''animal meat meal'' which was said to contain material mainly frompoultry or fish origin but OCCASIONALLY FROM BEEF SOURCES. the partiallythawed packs were tipped into an augur mixer which has a fully loadedcapacity of 6000lb and this would be approximately 15000 mink per day.

In the fall at pelting time the skinned carcasses of the mink are placed inlarge barrels which are left in the open to freeze. When full, a renderercollects ''for use in poultry feeds''.

Sections from the brains of the two Brecke TME inoculated cattle wereexamined and Marsh provided all the blocks from the 2nd steer for study atCVL and comparison with BSE. In general the vacuolar changes were moresevere than in most cases of BSE but very similar in distribution.Unfortunately material aken fro histopathology from those anials omittedrepresentaion of most of the brain stem. ...........

Wilbur Clarke (reference the Mission, Texas scrapie transmissiontransmission to cattle study) is now the State Veterinarian for Montanabased at Helena.

I was given confidential access to sections from the Clarke scrapie-cattletransmission experiment. Details of the experimental design were as suppliedpreviously by Dr. Wrathall (copy of relevant information appended). Only 3animals (2 inoculated with 2nd pass Suffolk scrapie and 1 inoculated withAngora goat passaged scrapie) showed clinical signs. Clinical signs werecharacterised by weakness, ''a stilted hindlimb gait'', disorientation,ataxia and, terminally, lateral recumbency. The two cattle from which Iexamined material were inocluated at 8 months of age and developed signs 36months pi (goat scrapie inoculum) and 49 months pi (one of the Suffolkscrapie inoculated) respectively. This latter animal was killed at 58 monthsof age and so the clinical duration was only 1 month. The neuropathology wassomewhat different from BSE or the Stetsonville TME in cattle. Vacuolarchanges were minimal, to the extent that detection REQUIRED CAREFULSEARCHING. Conversely astrocyte hypertrophy was a widespread and prominentfeature. The material requires DETAILED NEUROPATHOLOGICAL ASSESSMENT BUTWHETHER OR NOT THIS WILL BE DONE REMAINS A QUESTION.

Transmission Studies

Mule deer transmissions of CWD were by intracerebral inoculation andcompared with natural cases

{the following was written but with a single line marked through it ''firstpassage (by this route)}...TSS

resulted in a more rapidly progressive clinical disease with repeatedepisodes of synocopy ending in coma. One control animal became affected, itis believed through contamination of inoculum (?saline). Further CWDtransmissions were carried out by Dick Marsh into ferret, mink and squirrelmonkey. Transmission occurred in ALL of these species with the shortestincubation period in the ferret.

snip...

Appendix 3

VISIT TO USA - DR A E WRATHALL - INFO OH BSE AND SCRAPIE

1. Dr Clark lately of the Scrapie Research Unit, Mission Texas hassuccessfully transmitted ovine and caprine scrapie to cattle. Theexperimental results have not been published but there are plans to dothis. This work was initiated in 1978. A summary of it is:-

3. Prof. A Robertson gave a brief account of BSE. The US approach was toaccord it a very low profile indeed. Dr A Thiermann showed thepicture in the "Independent" with cattle being incinerated and thoughtthis was a fanatical incident to be avoided in the US at all costs.BSE was not reported in USA.

4. Scrapie incidents (ie affected flocks) have shown a dramatic increasesince 1978. In 1953 when the National Control Scheme was startedthere were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.

5. Scrapie agent was reported to have been isolated from a solitaryfetus.

6. A western blotting diagnostic technique (? on PrP) shows some promise.

7. Results of a questionnaire sent to 33 states on the subject of thenational sheep scrapie programme survey indicated

17/33 wished to drop it

6/33 wished to develop it

8/33 had few sheep and were neutral

Information obtained from Dr Wrathall's notes of a meeting of the U.S.Animal Health Association at Little Rock, Arkansas Nov. 1988.

It was, however, performed in the USA in 1979, when it was shown that cattleinoculated with the scrapie agent endemic in the flock of Suffolk sheep atthe United States Department of Agriculture in Mission, Texas, developed aTSE quite unlike BSE. 32 The findings of the initial transmission, thoughnot of the clinical or neurohistological examination, were communicated inOctober 1988 to Dr Watson, Director of the CVL, following a visit by DrWrathall, one of the project leaders in the Pathology Department of the CVL,to the United States Department of Agriculture. 33 The results were notpublished at this point, since the attempted transmission to mice from theexperimental cow brain had been inconclusive. The results of the clinicaland histological differences between scrapie-affected sheep and cattle werepublished in 1995. Similar studies in which cattle were inoculatedintracerebrally with scrapie inocula derived from a number ofscrapie-affected sheep of different breeds and from different States, werecarried out at the US National Animal Disease Centre. 34 The results,published in 1994, showed that this source of scrapie agent, thoughpathogenic for cattle, did not produce the same clinical signs of brainlesions characteristic of BSE.

To determine if sheep scrapie agent(s) in the United States would induce adisease in cattle resembling bovine spongiform encephalopathy, 18 newborncalves were inoculated intracerebrally with a pooled suspension of brainfrom 9 sheep with scrapie. Half of the calves were euthanatized 1 year afterinoculation. All calves kept longer than 1 year became severely lethargicand demonstrated clinical signs of motor neuron dysfunction that weremanifest as progressive stiffness, posterior paresis, general weakness, andpermanent recumbency. The incubation period was 14-18 months, and theclinical course was 1-5 months. The brain from each calf was examined forlesions and for protease-resistant prion protein. Lesions were subtle, but adisease-specific isoform of the prion protein was present in the brain ofall calves. Neither signs nor lesions were characteristic of those forbovine spongiform encephalopathy.

WE conclude that American sources of sheep scrapie are transmissible tocattle by direct intracerebral inoculation but the disease induced is NOTidentical to BSE as seen in the United Kingdom. While there weresimilarities in clinical signs between this experimental disease and BSE,there was no evidence of aggressiveness, hyperexcitability, hyperesthesia(tactile or auditory), or hyperemetria of limbs as has been reported for BSE(9). Neither were there extensive neurologic lesions, which are primary forBSE, such as severe vacuolation of neurons and neuropil or neuronal necrosisand gliosis. Although some vacuolation of neuropil, chromotolysis inneurons, and gliosis were seen in the brains of some affected calves, thesewere industinguishable from those of controls. Vacuolated neurons in the rednucleus of both challenged and normal calves were considered normal for thebovines as previously described (50).

PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS, andthe amount of PrP-res positively related to the length of the incubation....

snip...

WE also conclude from these studies that scrapie in cattle MIGHT NOT BERECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND SUGGESTTHAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting isnecessary to make a definitive diagnosis. THUS, undiagnosed scrapieinfection could contribute to the ''DOWNER-COW'' syndrome and could beresponsible for some outbreaks of transmissible mink encephalopathy proposedby Burger and Hartsough (8) and Marsh and harsough (52). ...

snip...

Multiple sources of sheep affected with scrapie and two breeds of cattlefrom several sources were used inthe current study in an effort to avoid asingle strain of either agent or host. Preliminary results from mouseinoculations indicate multiple strains of the agent were present in thepooled inoculum (unpublished data). ...

Transmission of the sheep scrapie to cattle was attempted in 1979 by usingintracerebral, intramuscular, subcutaneous, and oral routes of inoculationof 5, 8- to 11-month old cattlw with a homologous mixture of brain from 1affected sheep (61, 62). ONE of the 5 cattle develped neurologic signs 48months after inoculation. Signs were disorientation, incoordination, astiff-legged stilted gait, progressive difficulty in rising, and finally interminal recumbency. The clinical course was 2.5 months. TWO of the 5 cattlesimilarly inoculated with brain tissue from a goat with scrapie exhibitedsimilar signs 27 and 36 months after incoluation. Clinical courses were 43an 44 days. Brain lesions of mild gliosis and vacuolation and mouseinoculation data were insufficient to confirm a diagnosis of scrapie. Thiswork remained controversial until recent examination of the brains detectedPrP-res in all 3 cattle with neurologic disease but in none of theunaffected cattle (62). Results of these studies are similar to ours andunderscore the necessity of methods other than histopathology to diagnosescrapie infection in cattle. We believe that immunologic techniques fordetecting PrP-res currently provide the most sensitive and reliable way tomake a definitive diagnosis...

12/10/76AGRICULTURAL RESEARCH COUNCILREPORT OF THE ADVISORY COMMITTE ON SCRAPIEOffice NoteCHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to ScrapieA] The Problem

Scrapie is a natural disease of sheep and goats. It is a slowand inexorably progressive degenerative disorder of the nervous systemand it ia fatal. It is enzootic in the United Kingdom but not in allcountries.

The field problem has been reviewed by a MAFF working group(ARC 35/77). It is difficult to assess the incidence in Britain fora variety of reasons but the disease causes serious financial loss;it is estimated that it cost Swaledale breeders alone $l.7 M duringthe five years 1971-1975. A further inestimable loss arises from theclosure of certain export markets, in particular those of the UnitedStates, to British sheep.

It is clear that scrapie in sheep is important commercially andfor that reason alone effective measures to control it should bedevised as quickly as possible.

Recently the question has again been brought up as to whetherscrapie is transmissible to man. This has followed reports that thedisease has been transmitted to primates. One particularly luridspeculation (Gajdusek 1977) conjectures that the agents of scrapie,kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy ofmink are varieties of a single "virus". The U.S. Department ofAgriculture concluded that it could "no longer justify or permitscrapie-blood line and scrapie-exposed sheep and goats to be processedfor human or animal food at slaughter or rendering plants" (ARC 84/77)"The problem is emphasised by the finding that some strains of scrapieproduce lesions identical to the once which characterise the humandementias"

Whether true or not. the hypothesis that these agents might betransmissible to man raises two considerations. First, the safetyof laboratory personnel requires prompt attention. Second, actionsuch as the "scorched meat" policy of USDA makes the solution of theacrapie problem urgent if the sheep industry is not to suffergrievously.

Statement on Texas Cow With Central Nervous System SymptomsOn Friday, April 30 th , the Food and Drug Administration learned that a cowwith central nervous system symptoms had been killed and shipped to aprocessor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately beganan investigation. On Friday and throughout the weekend, FDA investigatorsinspected the slaughterhouse, the rendering facility, the farm where theanimal came from, and the processor that initially received the cow from theslaughterhouse.

FDA's investigation showed that the animal in question had already beenrendered into "meat and bone meal" (a type of protein animal feed). Over theweekend FDA was able to track down all the implicated material. Thatmaterial is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interestbecause cattle with bovine spongiform encephalopathy or BSE, also known as"mad cow disease," can exhibit such symptoms. In this case, there is no waynow to test for BSE. But even if the cow had BSE, FDA's animal feed rulewould prohibit the feeding of its rendered protein to other ruminant animals(e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informingthe firm that FDA will not object to use of this material in swine feedonly. If it is not used in swine feed, this material will be destroyed. Pigshave been shown not to be susceptible to BSE. If the firm agrees to use thematerial for swine feed only, FDA will track the material all the waythrough the supply chain from the processor to the farm to ensure that thefeed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian proteinout of animal feed for cattle and other ruminant animals. FDA establishedits animal feed rule in 1997 after the BSE epidemic in the U.K. showed thatthe disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is notallowed in feed for cattle or other ruminant animals. FDA's actionspecifying that the material go only into swine feed means also that it willnot be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closelywith the U.S. Department of Agriculture on all BSE issues. The animal feedrule provides crucial protection against the spread of BSE, but it is onlyone of several such firewalls. FDA will soon be improving the animal feedrule, to make this strong system even stronger.

####

http://www.fda.gov/bbs/topics/news/2004/NEW01061.html

IN TEXAS, we feed our cattle ruminant protein, and lots of it. but remember(the fda cannot seem to get this right)

Today the Food and Drug Administration announced the results of teststaken on feed used at a Texas feedlotthat was suspected of containing meat and bone meal from other domesticcattle -- a violation of FDA's 1997prohibition on using ruminant material in feed for other ruminants.Results indicate that a very low level ofprohibited material was found in the feed fed to cattle.

FDA has determined that each animal could have consumed, at most and intotal, five-and-one-half grams -approximately a quarter ounce -- of prohibited material. These animalsweigh approximately 600 pounds.

It is important to note that the prohibited material was domestic inorigin (therefore not likely to contain infectedmaterial because there is no evidence of BSE in U.S. cattle), fed at avery low level, and fed only once. Thepotential risk of BSE to such cattle is therefore exceedingly low, evenif the feed were contaminated.

According to Dr. Bernard Schwetz, FDA's Acting Principal DeputyCommissioner, "The challenge to regulatorsand industry is to keep this disease out of the United States. Oneimportant defense is to prohibit the use of anyruminant animal materials in feed for other ruminant animals. Combinedwith other steps, like U.S. Departmentof Agriculture's (USDA) ban on the importation of live ruminant animalsfrom affected countries, these stepsrepresent a series of protections, to keep American cattle free of BSE."

Despite this negligible risk, Purina Mills, Inc., is nonethelessannouncing that it is voluntarily purchasing all 1,222of the animals held in Texas and mistakenly fed the animal feedcontaining the prohibited material. Therefore,meat from those animals will not enter the human food supply. FDAbelieves any cattle that did not consumefeed containing the prohibited material are unaffected by this incident,and should be handled in the beef supplyclearance process as usual.

FDA believes that Purina Mills has behaved responsibly by firstreporting the human error that resulted in themisformulation of the animal feed supplement and then by working closelywith State and Federal authorities.

This episode indicates that the multi-layered safeguard system put intoplace is essential for protecting the foodsupply and that continued vigilance needs to be taken, by all concerned,to ensure these rules are followedroutinely.

FDA will continue working with USDA as well as State and local officialsto ensure that companies andindividuals comply with all laws and regulations designed to protect theU.S. food supply.

Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog,Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, NathalieLescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-PhilippeDeslysSummary The uncertain extent of human exposure to bovine spongiformencephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease(vCJD)--is compounded by incomplete knowledge about the efficiency of oralinfection and the magnitude of any bovine-to-human biological barrier totransmission. We therefore investigated oral transmission of BSE tonon-human primates. We gave two macaques a 5 g oral dose of brain homogenatefrom a BSE-infected cow. One macaque developed vCJD-like neurologicaldisease 60 months after exposure, whereas the other remained free of diseaseat 76 months. On the basis of these findings and data from other studies, wemade a preliminary estimate of the food exposure risk for man, whichprovides additional assurance that existing public health measures canprevent transmission of BSE to man.

Published online January 27, 2005

http://www.thelancet.com/journal/journal.isa

It is clear that the designing scientists must

also have shared Mr Bradley’s surprise at the results because all the dose

levels right down to 1 gram triggered infection.

http://www.bseinquiry.gov.uk/files/ws/s145d.pdf

2

6. It also appears to me that Mr Bradley’s answer (that it would take lessthan say 100

grams) was probably given with the benefit of hindsight; particularly if one

considers that later in the same answer Mr Bradley expresses his surprisethat it

could take as little of 1 gram of brain to cause BSE by the oral routewithin the

same species. This information did not become available until the "attackrate"

experiment had been completed in 1995/96. This was a titration experiment

designed to ascertain the infective dose. A range of dosages was used toensure

that the actual result was within both a lower and an upper limit within thestudy

and the designing scientists would not have expected all the dose levels totrigger

infection. The dose ranges chosen by the most informed scientists at thattime

ranged from 1 gram to three times one hundred grams. It is clear that thedesigning

scientists must have also shared Mr Bradley’s surprise at the resultsbecause all the

dose levels right down to 1 gram triggered infection.

http://www.bseinquiry.gov.uk/files/ws/s147f.pdf

Re: BSE .1 GRAM LETHAL NEW STUDY SAYS via W.H.O. Dr Maura Ricketts

[BBC radio 4 FARM news]

http://www.maddeer.org/audio/BBC4farmingtoday2_1_03.ram

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

2) Infectious dose:

To cattle: 1 gram of infected brain material (by oral ingestion)

http://www.inspection.gc.ca/english/sci/bio/bseesbe.shtml

NEW MAD COW STRAIN CALLED BASE, VERY SIMILAR TO SPORADIC CJD IN HUMANS;

Edited by Stanley B. Prusiner, University of California, San Francisco, CA,and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, aremammalian neurodegenerative disorders characterized by a posttranslationalconversion and brain accumulation of an insoluble, protease-resistantisoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human andanimal TSE agents exist as different phenotypes that can be biochemicallydifferentiated on the basis of the molecular mass of the protease-resistantPrPSc fragments and the degree of glycosylation. Epidemiological, molecular,and transmission studies strongly suggest that the single strain of agentresponsible for bovine spongiform encephalopathy (BSE) has infected humans,causing variant Creutzfeldt-Jakob disease. The unprecedented biologicalproperties of the BSE agent, which circumvents the so-called "speciesbarrier" between cattle and humans and adapts to different mammalianspecies, has raised considerable concern for human health. To date, it isunknown whether more than one strain might be responsible for cattle TSE orwhether the BSE agent undergoes phenotypic variation after naturaltransmission. Here we provide evidence of a second cattle TSE. The disorderwas pathologically characterized by the presence of PrP-immunopositiveamyloid plaques, as opposed to the lack of amyloid deposition in typical BSEcases, and by a different pattern of regional distribution and topology ofbrain PrPSc accumulation. In addition, Western blot analysis showed a PrPSctype with predominance of the low molecular mass glycoform and aprotease-resistant fragment of lower molecular mass than BSE-PrPSc.Strikingly, the molecular signature of this previously undescribed bovinePrPSc was similar to that encountered in a distinct subtype of sporadicCreutzfeldt-Jakob disease.

AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure3). There were 11 new infected and source flocks reported in March (Figure4) with a total of 51 flocks reported for FY 2005 (Figure 5). The totalinfected and source flocks that have been released in FY 2005 are 39 (Figure6), with 1 flock released in March. The ratio of infected and source flocksreleased to newly infected and source flocks for FY 2005 = 0.76 : 1. INaddition, as of March 31, 2005, 225 scrapie cases have been confirmed andreported by the National Veterinary Services Laboratories (NVSL), of which53 were RSSS cases (Figure 7). This includes 57 newly confirmed cases inMarch 2005 (Figure 8). Fourteen cases of scrapie in goats have been reportedsince 1990 (Figure 9). The last goat cases was reported in January 2005. Newinfected flocks, source flocks, and flocks released or put on clean-up plansfor FY 2005 are depicted in Figure 10. ...

WITH the MAY report, a scrapie case documented in a GOAT IN THE USA...TSS

SCRAPIE USA UPDATE MAY 2005

SCRAPIE has increased drastically since the report i posted in March 2005,with additional case in a goat;

SCRAPIE USA MONTHLY REPORT 2005

AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure3). There were 11 new infected and source flocks reported in March (Figure4) with a total of 51 flocks reported for FY 2005 (Figure 5). The totalinfected and source flocks that have been released in FY 2005 are 39 (Figure6), with 1 flock released in March. The ratio of infected and source flocksreleased to newly infected and source flocks for FY 2005 = 0.76 : 1. INaddition, as of March 31, 2005, 225 scrapie cases have been confirmed andreported by the National Veterinary Services Laboratories (NVSL), of which53 were RSSS cases (Figure 7). This includes 57 newly confirmed cases inMarch 2005 (Figure 8). Fourteen cases of scrapie in goats have been reportedsince 1990 (Figure 9). The last goat cases was reported in January 2005. Newinfected flocks, source flocks, and flocks released or put on clean-up plansfor FY 2005 are depicted in Figure 10. ...

IN light of Asante/Collinge et al findings that BSE transmission to the129-methionine genotype can lead to an alternate phenotype that isindistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;

Dormont*, and Jean-Philippe Deslys* et al, that The agent responsiblefor French iatrogenic growth hormone-linked CJD taken as a control isvery different from vCJD but is similar to that found in one case ofsporadic CJD and one sheep scrapie isolate;

http://www.pnas.org/cgi/content/full/041490898v1

Characterization of two distinct prion strainsderived from bovine spongiform encephalopathytransmissions to inbred mice

http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471

ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally, OFALL AGES...AND A DAMN WRITTEN CJD QUESTIONNAIRE ASKING REAL QUESTIONS PERTAINING TO ROUTE AND SOURCE OF ALL STRAINS, WITH A WRITTEN COPY GOING TO FAMILY...accept nothing less, just say NO...!